Abstract
BACKGROUND: Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory lesion, especially in orbit. EAF is believed to be related to IgG4-related disease (IgG4-RD), but the clinical manifestations of systemic involvement are relatively rare and easy to be confused with tumors or other inflammatory diseases. Histopathological examination is the most important way of its diagnosis and differentiation. CASE PRESENTATION: We presented a 55-year-old female patient presented with recurrent swelling of the right lower eyelid for more than 2 months. The pathological diagnosis was EAF. Positive immunostaining for IgG, CD34, κ, and λ, while negative immunostaining for IgG4. CONCLUSIONS: Complete surgical resection is the preferred treatment, histopathological examination is the main diagnostic standard.